Tumores Ósseos Malignos Flashcards
Qual a faixa etária mais comumente afetada pelo osteossarcoma primário?
primary high-grade osteosarcoma occurs most commonly in the second decade of life.
Qual o tumor ósseo não hematológico mais comum?
Osteossarcoma
Qual o pico de incidência do osteossarcoma parosteal?
Parosteal osteosarcoma has a peak incidence in the third and fourth decades
Em que idade geralmente ocorrem os osteossarcomas secundários?
Secondary osteosarcomas (e.g., those that occur in the setting of Paget disease or previous radiation therapy) are more common in older individuals.
Quais patologias estão relacionadas a maior chance de desenvolvimento de osteossarcoma?
Osteosarcoma may be more common in patients with the hereditary form of retinoblastoma, Rothmund-Thomson syndrome, and Li-Fraumeni syndrome.
Quais os locais mais frequentes de desenvolvimento de osteossarcoma?
Most primary osteosarcomas occur at the sites of the most rapid bone growth, including the distal femur, the proximal tibia, and the proximal humerus.
Qual a queixa deve deixar o médico atento para o risco de osteossarcoma?
Night pain may be an important clue to the true diagnosis; however, only about 25% of patients experience this phenomenon.
Quais são os achados radiográficos dos osteossarcomas?
he most common appearance is that of an aggressive lesion in the metaphysis of a long bone. Approximately 10% are primarily diaphyseal, and less than 1% are primarily epiphyseal. Although the lesion can be either predominantly blastic or predominantly lytic, more commonly areas of bone production and bone destruction are present. The lesion usually is quite permeative, and the borders are ill defined.
Quais são as reações periosteais encontradas nas radiografias dos osteossarcomas?
Periosteal reaction may take the form of a “Codman triangle,” or it may have a “sunburst” or “hair-on-end” appearance.
Qual o local mais comum de metástase por osteossarcoma?
Pulmões
Quais são as subcategorias dos osteossarcomas primarios?
Primary osteosarcomas are subcategorized as conventional osteosarcoma, low-grade intramedullary osteosarcoma, parosteal osteosarcoma, periosteal osteosarcoma, high-grade surface osteosarcoma, telangiectatic osteosarcoma, and small cell osteosarcoma.
Quais são as caracteristicas dos osteossarcomas periosteais?
Periosteal osteosarcoma is an intermediate-grade malignancy that arises on the surface of the bone. The most common locations are the diaphyses of the femur and tibia.
Qual a localização mais frequente dos osteossarcomas parosteais?
It has a peculiar tendency to occur as a lobulated ossified mass on the posterior aspect of the distal femur.
Como diferenciar miosite ossificante de osteossarcoma parosteal pela TC?
The ossification in myositis ossificans is more mature at the periphery of the lesion, whereas the center of a parosteal osteosarcoma is more heavily ossified.
Qual subtipo de osteossarcoma é o mais raro?
High-grade surface osteosarcoma is the least common type of osteosarcoma.
Qual é a aparencia radiográfica do osteossarcoma teleangectásico?
Telangiectatic osteosarcoma is a purely lytic lesion. On a radiograph, it can have an invasive appearance or it can have a ballooned appearance similar to that of an aneurysmal bone cyst.
Quais são os fatores mais associados ao desenvolvimento de osteossarcoma secundário?
The most common factors associated with secondary osteosarcomas include Paget disease and previous radiation therapy. The incidence of osteosarcoma in Paget disease is approximately 1% and may be higher (5% to 10%) for patients with advanced polyostotic disease. Pelvis is the most common location.
Other conditions that have been reported to be associated with secondary osteosarcomas include fibrous dysplasia, bone infarcts, osteochondromas, chronic osteomyelitis, melorheostosis, and osteogenesis imperfecta;
Como era e como é a taxa de sobrevida de longo termo dos pacientes com osteossarcoma?
Before the advent of multiple-agent chemotherapy, the prognosis for patients with osteosarcoma was dismal. Despite treatment consisting of wide or radical amputation, approximately 80% of patients died as a result of distant metastases, usually within 2 years. With today’s multiple-agent chemotherapy regimens and appropriate surgical treatment, most series report long-term survival of 60% to 75% for patients with high-grade osteosarcoma without metastases at initial presentation and 90% for those with low-grade lesions.
Qual a porcentagem aproximada de pacientes que se apresentam com metástase no momento do diagnóstico?
Approximately 15% of patients with osteosarcoma have detectable pulmonary metastases at the time of diagnosis.
Qual o fator prognóstico mais importante nos osteossarcomas?
Presença ou não de metástase (skip, pulmonar ou óssea)
Qual a relação da localização do osteossarcoma com seu prognóstico?
Skeletal location also is thought to be important because patients with more proximal tumors do worse than patients with more distal tumors.
Como é realizado o tratamento dos osteossarcomas?
Currently, at most musculoskeletal oncology centers, the treatment of high-grade osteosarcoma consists of neoadjuvant chemotherapy, wide or radical surgery (resection or amputation), and adjuvant chemotherapy.
Como avaliar se um tumor respondeu bem à quimioterapia neoadjuvante?
The histological response of the primary tumor to neoadjuvant chemotherapy has been shown to be a good predictor of long-term survival. Greater than 90% tumor necrosis indicates a very good prognosis.
Qual é a taxa de recorrência dos osteossarcomas?
About 10% of patients have local recurrence after wide resection or wide amputation.
Quais são os fatores de pior prognóstico para pacientes com recidiva dos osteossarcomas?
Poor prognostic factors include rapid relapse after completion of the initial treatment, many (more than eight) pulmonary nodules, large (>3 cm) pulmonary nodules, and unresectable pulmonary nodules. Patients with a few, small, resectable pulmonary nodules that occur late may have a 40% chance of cure with aggressive treatment.
Em quais faixas etárias ocorrem os picos de incidencia dos condrossarcomas?
It occurs over a broad age range, with peaks between 40 and 60 years for primary chondrosarcoma and between 25 and 45 years for secondary chondrosarcoma.
Quais são os locais mais comumentes afetados pelos condrossarcomas?
Most are located in a proximal location such as the pelvis, proximal femur, and proximal humerus. Although chondrosarcomas rarely occur in the hand, they are the most common primary malignancy of bone in this location.
Quais são as entidades benignas relacionadas aos condrossarcomas secundarios?
Secondary chondrosarcomas arise at the site of a preexisting benign cartilage lesion. They occur most frequently in the setting of multiple enchondromas and multiple hereditary exostoses. In Ollier disease (multiple enchondromas) the incidence of malignancy (most commonly chondrosarcoma) is approximately 25% by age 40 years, and in patients with Mafucci syndrome (multiple enchondromas with soft tissue hemangiomas) the incidence may be even higher.
Other conditions that have been reported to be associated with secondary chondrosarcoma include synovial chondromatosis, chondromyxoid ibroma, periosteal chondroma, chondroblastoma, previous radiation treatment, and ibrous dysplasia.
Quais são as características radiográficas dos condrossarcomas?
The radiographic appearance of chondrosarcoma frequently is diagnostic. Similar to enchondroma, it is a lesion arising in the medullary cavity with irregular matrix calcification. The pattern of calcification has been described as “punctate,” “popcorn,” or “comma shaped.” Compared with enchondroma, however, chondrosarcoma has a more aggressive appearance with bone destruction, cortical erosions, periosteal reaction, and, rarely, a soft tissue mass.
Qual a espessura do “cap” do osteocondroma deve levantar a suspeita de um condrossarcoma em um paciente esqueléticamente maduro?
Finally, the size of the cartilaginous cap of an osteochondroma, as evaluated with CT or MRI, is important in evaluating the possibility of a secondary chondrosarcoma. If the cartilaginous cap is larger than 2 cm in a skeletally mature patient, a secondary chondrosarcoma must be considered.
Quais são as caracteristicas dos condrossarcomas de células claras?
Clear cell chondrosarcoma has a strong tendency to arise in an epiphysis (especially the proximal femur). It may have benign radiographic features and can be confused with chondroblastoma or giant cell tumor.
Qual é o tratamento para os condrossarcomas de baixo e alto grau?
Extended curettage is considered adequate treatment only for low-grade lesions that are confined within the medullary canal. Those with soft tissue extension should be treated similar to high-grade lesions. The treatment of high-grade chondrosarcoma is wide or radical resection or amputation.
Qual a sobrevida dos pacientes com condrossarcoma?
Patients with low-grade lesions have been reported to have a greater than 90% 10-year survival rate, whereas patients with high-grade conventional chondrosarcoma are reported to have a 20% to 40% 10-year survival rate. he 5-year survival rate is less than 15% for patients with dediferentiated chondrosarcoma, with most deaths occurring in the irst 2 years.
Qual é o tumor osseo mais comum em crianças < 10 anos?
Ewing
Qual é a localização mais comum do sarcoma de Ewing?
The most common locations include the metaphyses of long bones (often with extension into the diaphysis) and the flat bones of the shoulder and pelvic girdles.
Em qual população o sarcoma de Ewing é raro?
Ewing sarcoma is exceedingly rare in individuals of African descent.
Além da dor, quais são os outros sintomas do sarcoma de Ewing?
In addition to pain, patients also may have fever, erythema, and swelling, suggesting osteomyelitis.
Como é a apresentação radiografica do sarcoma de Ewing?
Classically, Ewing sarcoma appears radiographically as a destructive lesion in the diaphysis of a long bone with an “onion skin” periosteal reaction. In reality, Ewing sarcoma more often originates in the metaphysis of a long bone but frequently extends for a considerable distance into the diaphysis.
Qual é o local mais comum de metástases no sarcoma de Ewing? E o segundo?
Pulmão e osso